Features of Autoimmune Hepatitis in Egyptian Children

Background and Aim: Autoimmune hepatitis (AIH) is a progressive inflammatory liver disorder mainly affecting female and its diagnosis is based on a series of positive and negative criteria. The aim of this study is to define the clinical, biochemical features and the response to immunosuppression in children with AIH and also to compare the performance of the International Autoimmune Hepatitis Group (IAIHG) revised original scoring system and the simplified scoring system. Patients and Methods: Data of 30 children (22 female and 8 male) with AIH have been analyzed for their clinical, serological, histological profile and their response to treatment. We also re-analyzed their clinical features using the IAIHG revised original scoring system and the simplified scoring system. Results: The median age at diagnosis was 8 years (range, 3-12 years). Clinical features at presentation included jaundice (90%), hepatomegaly (80%), splenomegaly (43%), pallor (10%), lower limb edema (6.6%) and ascites (3%). Acute hepatic like illness was the first presentation in (66.7%). Initial laboratory investigation revealed high serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), and serum immunoglobulin G (IgG) in all patients. Antinuclear antibodies (ANAs), anti-smooth muscle antibody (SMA), anti-soluble liver antigen (SLA), and perinuclear antineutrophil cytoplasmic antibodies (pNCAN) were positive in 36.7%, 86.7%, 16.6%, 30% patients respectively. No patient was seropositive for anti-liver kidney microsome antibody (antiLKM). Liver biopsy was performed in 26 patients. Interface hepatitis was present in 77% patients, rosette formation in 34.6%, biliary changes in 69%, and lymphoblastic infiltrates in all patients. Cirrhosis was present in 30.8% patients and fibrosis in 96% patients. All patients received steroids as the initial therapy and in 22 of them azathioprine was added to start steroid withdrawal. Complete response to treatment was observed in 33.3% patients; partial response in 10%, and 56.7% had relapses. The sensitivity of original revised scoring system was 100% versus 88.5% for the simplified scoring system for diagnosis AIH among our patients. Concordance between the original revised and the simplified scores for a probable or definite diagnosis of AIH was 38.5% and 19.2%, respectively. Correspondence to: Dr. Ahmad Y. Rezk, Nehal El-Koofy, Pediatr. Dept., Fac. of Medicine, Cairo Univ., Cairo, Egypt. Conclusion: Survival in AIH apparently good with early diagnosis and treatment. The revised original score system performed better among our patients than the simplified score system.